Dept. of Neurosciences, University of Padova, Padova, Italy.
Dept. of Neurosciences, University of Padova, Padova, Italy.
J Neuroimmunol. 2019 May 15;330:178-180. doi: 10.1016/j.jneuroim.2019.01.017. Epub 2019 Jan 29.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with anti-neurofascin-155 antibodies is a subgroup of CIDP with tremor and poor response to intravenous immunoglobulins. A 23-year-old male presented with a 6-month history ataxic-stepping gait, stocking tactile hypoesthesia, areflexia, tremor at limbs and tongue. Neurophysiology and cerebrospinal fluid analysis supported the diagnosis of CIDP. Tongue EMG was negative. Serum was positive for neurofascin-155 IgG4. His symptoms improved with intravenous methylprednisolone and then immunoglobulins, but not the tremor. Neurofascin-155 antibodies binding to cerebellar neurons suggests its central origin. This is the first neurofascin-155 antibody-seropositive patient with also tongue tremor, who is candidate to rituximab.
抗神经束蛋白 155 抗体相关性慢性炎症性脱髓鞘性多发性神经病（CIDP）是 CIDP 的一个亚型，伴有震颤和对静脉注射免疫球蛋白反应不佳。一名 23 岁男性出现共济失调步态 6 个月，袜子样触觉减退，反射消失，四肢和舌震颤。神经生理学和脑脊液分析支持 CIDP 的诊断。舌肌电图为阴性。血清神经束蛋白 155 IgG4 阳性。他的症状在静脉用甲基强的松龙和免疫球蛋白后改善，但震颤未改善。神经束蛋白 155 抗体与小脑神经元结合提示其中枢起源。这是首例神经束蛋白 155 抗体阳性伴有舌震颤的患者，适合利妥昔单抗治疗。
